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Hypoplastic left heart syndrome

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https://www.readbyqxmd.com/read/28711428/-congenital-heart-disease-mortality-in-spain-during-a-10-year-period-2003-2012
#1
Javier Pérez-Lescure Picarzo, Margarita Mosquera González, Pello Latasa Zamalloa, David Crespo Marcos
INTRODUCTION AND OBJECTIVES: Congenital heart disease is a major cause of infant mortality in developed countries. In Spain, there are no publications at national level on mortality due to congenital heart disease. The aim of this study is to analyse mortality in infants with congenital heart disease, lethality of different types of congenital heart disease, and their variation over a ten-year period. METHODS: A retrospective observational study was performed to evaluate mortality rate of children under one year old with congenital heart disease, using the minimum basic data set, from 2003 to 2012...
July 12, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28698113/patient-specific-biomechanical-model-of-hypoplastic-left-heart-to-predict-post-operative-cardio-circulatory-behaviour
#2
Elena Cutrì, Alessio Meoli, Gabriele Dubini, Francesco Migliavacca, Tain-Yen Hsia, Giancarlo Pennati
Hypoplastic left heart syndrome is a complex congenital heart disease characterised by the underdevelopment of the left ventricle normally treated with a three-stage surgical repair. In this study, a multiscale closed-loop cardio-circulatory model is created to reproduce the pre-operative condition of a patient suffering from such pathology and virtual surgery is performed. Firstly, cardio-circulatory parameters are estimated using a fully closed-loop cardio-circulatory lumped parameter model. Secondly, a 3D standalone FEA model is build up to obtain active and passive ventricular characteristics and unloaded reference state...
July 8, 2017: Medical Engineering & Physics
https://www.readbyqxmd.com/read/28689327/in-utero-evidence-of-impaired-somatic-growth-in-hypoplastic-left-heart-syndrome
#3
Jourdan E Triebwasser, Marjorie C Treadwell
We hypothesized that fetuses with hypoplastic left heart syndrome (HLHS) have impaired growth compared to expected growth for gestational age. This is a retrospective cohort study including singleton fetuses with isolated HLHS identified from a single, referral center's ultrasound database. To account for variable timing of ultrasounds, z-scores for gestational age were assigned for each biometric parameter. We identified 169 fetuses, of which 96 had more than one ultrasound. The median number of ultrasound evaluations per fetus was 2 (range 1-5)...
July 8, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28687711/the-optimal-timing-of-stage-2-palliation-for-hypoplastic-left-heart-syndrome-an-analysis-of-the-pediatric-heart-network-single-ventricle-reconstruction-trial-public-dataset
#4
James M Meza, Edward J Hickey, Eugene H Blackstone, Robert D B Jaquiss, Brett R Anderson, William G Williams, Sally Cai, Glen S Van Arsdell, Tara Karamlou, Brian W McCrindle
Background -In infants requiring three-stage single ventricle palliation for hypoplastic left heart syndrome, attrition after the Norwood procedure remains significant. The effect of the timing of stage-2-palliation (S2P), a physician-modifiable factor, on long term survival is not well understood. We hypothesized that an optimal interval between the Norwood and S2P that both minimizes pre-S2P attrition and maximizes post-S2P survival exists and is associated with individual patient characteristics. Methods -The NIH/NHLBI Pediatric Heart Network Single Ventricle Reconstruction Trial public dataset was used...
July 7, 2017: Circulation
https://www.readbyqxmd.com/read/28684000/management-of-tricuspid-regurgitation-in-patients-with-hypoplastic-left-heart-syndrome
#5
Frank A Pigula, Bret Mettler
Tricuspid valve (TV) performance is critical for palliation of hypoplastic left heart syndrome. We will review current TV repair techniques, outcomes, and novel approaches.
April 2017: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28683514/endocardial-fibroelastosis-of-the-left-ventricle-affects-right-ventricular-performance-in-fetuses-with-hypoplastic-left-heart-syndrome-a-prospective-study-using-m-mode-pw-and-tissue-doppler-techniques
#6
Oliver Graupner, Christian Enzensberger, Larissa Wieg, Jan Degenhardt, Aline Wolter, Markus Khalil, Dietmar Schranz, Can Yerebakan, Astrid Doelle, Johannes Herrmann, Roland Axt-Fliedner
Purpose Myocardial function (MF) of the systemic right ventricle (RV) influences the postnatal course of neonates with hypoplastic left heart syndrome (HLHS). Our study examines whether the presence of endocardial fibroelastosis of the left ventricle (LV EFE) influences MF of the RV in HLHS fetuses. Materials and Methods A prospective study was conducted including 10 controls (group 1), 10 HLHS fetuses with (group 2) and 10 without LV EFE (group 3) - all matched for gestational age. M-mode was used to assess tricuspid plane systolic excursion (TAPSE) and the shortening fraction (SF)...
July 6, 2017: Ultraschall in der Medizin
https://www.readbyqxmd.com/read/28677480/preoperative-venovenous-ecmo-in-an-infant-with-late-diagnosis-of-hypoplastic-left-heart-syndrome
#7
Lena K Eschenbach, Jelena Kasnar-Samprec, Kilian Ackermann, Christian Schreiber, Rudiger Lange, Julie Cleuziou
Late diagnosis and late referral for the Norwood stage 1 procedure in patients with hypoplastic left heart syndrome is rare and associated with a higher mortality. We present a case of a cyanotic almost five-week-old infant with hypoplastic left heart syndrome, highly restrictive foramen ovale, and patent ductus arteriosus, who was bridged with venovenous extracorporeal membrane oxygenation to the Norwood stage 1 procedure.
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28667405/utilizing-hybrid-techniques-to-maximize-clinical-outcomes-in-congenital-heart-disease
#8
REVIEW
David W Bearl, Gregory A Fleming
PURPOSE OF REVIEW: The past couple of decades have brought tremendous advances to the field of pediatric and adult congenital cardiology. Recent collaborations between interventional cardiologists and cardiac surgeons have sparked novel innovative hybrid procedures that provide potentially safer, faster, and less invasive alternatives to the management of many diseases. This paper will review the most recent advances in hybrid interventions in the field of pediatric and adult congenital cardiology...
August 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28661741/hypoplastic-left-heart-syndrome-the-case-against-palliative-surgery
#9
Gwen E Erkonen, Robert A Hanfland
No abstract text is available yet for this article.
July 2017: American Journal of Bioethics: AJOB
https://www.readbyqxmd.com/read/28661739/growing-discomfort-with-comfort-care-for-hypoplastic-left-heart-syndrome-why-we-should-still-defer-to-parental-wishes
#10
Erin A Paul, Elizabeth M Kohlberg, Kristina Orfali
No abstract text is available yet for this article.
July 2017: American Journal of Bioethics: AJOB
https://www.readbyqxmd.com/read/28661733/the-ethics-of-treatment-for-hypoplastic-left-heart-syndrome-hlhs
#11
Jeffrey P Spike
No abstract text is available yet for this article.
July 2017: American Journal of Bioethics: AJOB
https://www.readbyqxmd.com/read/28661728/may-we-take-our-baby-with-hypoplastic-left-heart-syndrome-home
#12
Mary Devereaux, Alexander A Kon
No abstract text is available yet for this article.
July 2017: American Journal of Bioethics: AJOB
https://www.readbyqxmd.com/read/28643402/clinical-findings-in-right-ventricular-noncompaction-in-hypoplastic-left-heart-syndrome
#13
Monique M Gardner, Meryl S Cohen
BACKGROUND: Noncompaction is a poorly understood form of cardiomyopathy that typically affects the left ventricle and may be associated with congenital heart disease. Right ventricular noncompaction (RVNC) may occur when the left ventricle is affected but is rarely seen in isolation. RVNC may have clinical significance affecting surgical and long-term outcomes. We describe the diagnosis and clinical course in three patients at our institution. METHODS: We performed a retrospective review of patients diagnosed with RVNC over a 12-month period at our institution and reviewed their imaging and clinical course...
June 23, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28631156/diagnosis-of-secondary-pulmonary-lymphangiectasia-in-congenital-heart-disease-a-novel-role-for-chest-ultrasound-and-prognostic-implications
#14
Christopher Z Lam, Tanmay Anant Bhamare, Tamadhir Gazzaz, David Manson, Tilman Humpl, Mike Seed
BACKGROUND: Secondary pulmonary lymphangiectasia is a complication of congenital heart disease that results from chronic pulmonary venous obstruction. OBJECTIVES: We aimed to evaluate the performance of chest ultrasound (US) in diagnosing secondary pulmonary lymphangiectasia and to review the clinical course of children with secondary pulmonary lymphangiectasia. MATERIALS AND METHODS: Chest US was performed on 26 children with hypoplastic left heart syndrome, total anomalous pulmonary venous connection or cor triatriatum in a prospective observational study...
June 19, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28627006/parents-perceptions-during-the-transition-to-home-for-their-child-with-a-congenital-heart-defect-how-can-we-support-families-of-children-with-hypoplastic-left-heart-syndrome
#15
Sarita March
PURPOSE: The aim of the study was to explore the literature related to transitions in healthcare between the hospital and home that caregivers experience with a child who has a congenital heart defect (CHD), specifically related to hypoplastic left heart syndrome (HLHS). DESIGN AND METHODS: A systematic literature review was conducted searching OVID Medline, CINAHL, and PubMed to discover the caregivers' perceptions on their transitions between hospital care and home care of their child with a CHD...
June 18, 2017: Journal for Specialists in Pediatric Nursing: JSPN
https://www.readbyqxmd.com/read/28608148/hypoplastic-left-heart-syndrome-sequencing-reveals-a-novel-notch1-mutation-in-a-family-with-single-ventricle-defects
#16
Matthew D Durbin, Adrian G Cadar, Charles H Williams, Yan Guo, David P Bichell, Yan Ru Su, Charles C Hong
Hypoplastic left heart syndrome (HLHS) has been associated with germline mutations in 12 candidate genes and a recurrent somatic mutation in HAND1 gene. Using targeted and whole exome sequencing (WES) of heart tissue samples from HLHS patients, we sought to estimate the prevalence of somatic and germline mutations associated with HLHS. We performed Sanger sequencing of the HAND1 gene on 14 ventricular (9 LV and 5 RV) samples obtained from HLHS patients, and WES of 4 LV, 2 aortic, and 4 matched PBMC samples, analyzing for sequence discrepancy...
June 12, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28583678/hospital-inpatient-costs-for-single-ventricle-patients-surviving-the-fontan-procedure
#17
Li Huang, Chris Schilling, Kim M Dalziel, Siyu Xie, David S Celermajer, John J McNeil, David Winlaw, Tim S Hornung, Dorothy J Radford, Leeanne E Grigg, Andrew Bullock, Gavin R Wheaton, Robert N Justo, Jayne Blake, Rachel Bishop, Karin Du Plessis, Yves d'Udekem
We estimated the inpatient resource use for a Fontan patient from birth to adulthood and explored factors that might induce cost differences (2014 US dollar). Inpatient costing records from 4 hospitals with greatest numbers of Fontan patients in Australia and New Zealand were linked with the Fontan registry database. Inpatient records between July 1995 and September 2014 for 420 Fontan patients were linked, and the most frequent primary diagnoses were hypoplastic left heart syndrome (20.7%), tricuspid atresia (19...
August 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28583211/parenting-a-child-at-home-with-hypoplastic-left-heart-syndrome-experiences-of-commitment-of-stress-and-of-love
#18
Annie M Cantwell-Bartl, James Tibballs
OBJECTIVE: To evaluate the experiences of parenting a child with hypoplastic left heart syndrome after the child has been discharged home from hospital. DESIGN: A study of the parents' experiences using face-to-face interviews and psychometric measures with parents whose child had survived stage surgery. SETTING: Parents were interviewed within the home environment or within the hospital if that was their choice. SUBJECTS: A total of 29 parents (16 mothers and 13 fathers) of surviving children...
June 6, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28578168/neonatal-outcomes-in-fetuses-with-cardiac-anomalies-and-the-impact-of-delivery-route
#19
Laura I Parikh, Katherine L Grantz, Sara N Iqbal, Chun-Chih Huang, Helain J Landy, Melissa H Fries, Uma M Reddy
BACKGROUND: Congenital fetal cardiac anomalies compromise the most common group of fetal structural anomalies. Several previous reports analyzed all types of fetal cardiac anomalies together without individualized neonatal morbidity outcomes based on cardiac defect. Mode of delivery in cases of fetal cardiac anomalies varies greatly as optimal mode of delivery in these complex cases is unknown. OBJECTIVE: We sought to determine rates of neonatal outcomes for fetal cardiac anomalies and examine the role of attempted route of delivery on neonatal morbidity...
May 31, 2017: American Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28568976/percutaneous-obliteration-of-left-ventricular-cavity-to-eliminate-aortic-regurgitation-and-presumed-coronary-steal-in-an-infant-with-hypoplastic-left-heart-syndrome
#20
Howaida G El-Said, Charissa R Pockett, John W Moore
The phenomenon of coronary steal is well known in the setting of HLHS (Hypoplastic Left Heart Syndrome) early after the Classical Norwood Operation. We report a rare case of an infant with HLHS [Severe Aortic Stenosis (AS), Mitral Stenosis (MS) and small Left Ventricle (LV)], who developed aortic regurgitation and presumed coronary steal late after the Sano Modification of the Norwood Procedure. Coronary steal developed secondary to progressive aortic and mitral regurgitation and resulted in poor right ventricular function and severe tricuspid regurgitation...
June 1, 2017: Catheterization and Cardiovascular Interventions
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